Eccrine Carcinoma

Idrissi Serhrouchni et al. Diagnostic Pathology 2013, 8:15 http://www.diagnosticpathology.org/content/8/1/15 CASE REPORT Open Access Eccrine carcinoma : a rare cutaneous neoplasm Karima Idrissi Serhrouchni1*, Taoufiq Harmouch1, Laila Chbani1, Hind El Fatemi1, Mohammed Sekal1, Nawal Hammas1, Meriem Soughi2, Loubna Benchat2 and Afaf Amarti1 Abstract Eccrine carcinoma is an extremely rare malignancy of the skin with few well documented cases reported in the literature. It is frequently found on the lower extremities, and it equally affects both sexes in the sixth and seventh decade. In our case, we present a 46- year-old female with a recurring exophytic tumor on the right lower extremity, without local extension. The initial tumor was biopsied, excised and diagnosed as an eccrine carcinoma. Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/ vs/3568051328673318. Keywords: Eccrine carcinoma, Sweat gland tumor, Mammary gland Background Tumor markers as like as CA 19–9, CA 125, CA 15–3 Malignant cutaneous adnexal neoplasms are a large and va- were normal. MRI of the leg showed a subcutaneous ried group, in particular eccrine carcinoma. They are one of infiltrative process coming into contact with the muscle the most challenging areas of Dermatopathology [1]. fascia. Eccrine and apocrine neoplasms present a bewildering The rest of the radiological examination, including radiog- array of morphologies which often defy precise classifi- raphy of the lung, abdominopelvic ultrasonography, com- cation [2]. puted tomographic thoraco-abdomino-pelvic scan, was The purpose of this case is to discuss the most common normal. problems concerning the classification of this rare neoplasm The mammography and ultrasound examination objecti- and report the aim of the immunohistochemical profiles in fied a 4 mm measuring benign cystic lesion of the left breast differential diagnosis between a primitive eccrine carcinoma classified ACR 3, requiring supervision during six months. of the skin and a secondary neoplasm. After biopsy of the lesion, a surgical excision with clear margins was performed. On gross examination, the tumor was 15 cm in size, Case report bourgeoning, erythematous and heterogeneous with In July 2012, a 45-year-old Moroccan woman presented areas of necrosis and haemorrhage. to the department of Dermatology of Hassan II Univer- Microscopic examination of the specimen revealed an sity Hospital of Fez with a 25-year-history of primary undifferentiated malignant proliferation with nodular infertility, and an 18-month-history of an exophytic mass architecture, located in the deep dermis (Figure 2). at the posterior right lower extremity, gradually increas- Tumor cells made clear cytoplasm with an hyperchro- ing in size. She was otherwise healthy and had no matic nucleus and a high mitotic activity (Figure 3). systemic symptoms. Physical examination showed an ul- Immunohistochemical analysis showed positive stain- cerative and bourgeoning mass of 15 cm with bleeding ing for cytokeratine and progesterone receptor. Cytoke- and purulent features (Figure 1). Biological tests ratine 7 was weakly positive and Ki67 was estimated at included a complete blood count, routine blood and 95% (Figure 4). urine chemistry were normal, except for elevated LDH. In contrast, the tumor cells were not reactive to cyto- * Correspondence: [email protected] keratine 20, estrogen receptor, LCA, CD10, S 100 1Department of Pathology, Hassan II University Hospital, Fez 30000, Morocco Full list of author information is available at the end of the article © 2013 Idrissi Serhrouchni et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Idrissi Serhrouchni et al. Diagnostic Pathology 2013, 8:15 Page 2 of 4 http://www.diagnosticpathology.org/content/8/1/15 sweat gland adenoma [5]. Such an approach, however, poses several problems; for example, (1) some carcinomas have no benign counterpart and do not fit the scheme (ductal carcinoma, adenoid cystic carcinoma, and mucinous carcinoma); (2) poorly differentiated carcinomas can be diagnosed only when a contiguous adenoma is found histologically; (3) histologic classification can be very complicated because adenomas are nume- rous and their classification is complex; and (4) terminology includes unusual and difficult terms, deriving from the terminology used for adenomas (malignant acrospiroma, porocarcinoma, hidradenocarcinoma, malignant cylindroma, malignant spiradenoma, and syrin- gocystadenocarcinoma) [6-9]. Recently, a classification of sweat gland carcinomas Figure 1 Clinical picture showed an ulcerative and bourgeoning mass of 15 cm with bleeding and purulent designed based on the classification of breast carcinomas features on the posterior right lower extremity. has been tentatively proposed [9]. Finally, recent studies have classified sweat gland carcinomas into eccrine and apocrine tumors [8,9]. In protein, HMB45, desmin, H-caldesmon, CD 99, CD 117, addition, no established authentic criteria are available CD 31 and CD 34. for differentiation of an eccrine from an apocrine tumor Based on these data, the diagnosis of primary cutane- [10]. Moreover, both eccrine and apocrine forms seem ous eccrine carcinoma was retained. to exist in some categories [5,9,11,12]. The correct identification of the origin of tumor is of Discussion utmost importance for the determination of appropriate Eccrine glands are directly developed from the em- therapy and prognosis. Immunohistochemistry has bryonic epidermis in the early months of fetal develop- proved to be a useful adjunct for this purpose [13]. ment [1]. They are widely distributed almost everywhere The cells in the excretory coil of eccrine gland sweat on the skin [2]. The topographic distribution of adnexal express positivity for low molecular weight keratin, epi- structures also offers insight into the logical classifica- thelial membrane antigen (EMA) and carcinoembryonic tion of adnexal neoplasms [3,4]. antigen (CEA), as well as S100 protein in the basal layer Microscopy alone is insufficient to establish eccrine only. The myoepithelial cell layer may be highlighted by lineage neoplasm because there are none specific micro- smooth muscle actin (SMA), p63 and calponin. Acrosy- scopic features [3]. ringeal cells stain for high molecular weight keratin and Some problems are related to the classification of cytokeratin 14. A subpopulation of cells also expresses sweat gland carcinomas, which are currently classified positivity to bcl-2 [2]. Some eccrine carcinomas are posi- on the basis of the corresponding classification of benign tif to estrogen and progesterone receptors wich has an important clinical implications, as affected patients may be partially treated with hormonal therapy [14]. The Ki- 67 and p53 may be used to differentiate benign from malignant lesions [15]. Clinically, eccrine carcinoma must be considered in the differential diagnosis of patients older than fifty years with long standing tumors in the limbs, including basal cell carcinoma (BCC), Paget’s disease, melanoma, metastatic cancer [16], inflammatory, lymphocytic and vascu- lar lesions [17]. The histopathology of mucinous eccrine carcinoma of the skin is analogous to that of its counterpart in the breast. Consequently, it is sometimes difficult to exclude a metastatic disease [15,18]. In a comparison between Figure 2 An undifferentiated malignant proliferation with primary sweat gland tumors and metastatic breast car- nodular architecture, located in the deep dermis (hematoxylin- cinoma to skin, Busam et al. [13,19] found that the use eosin, original magnification: x 100). of antibodies against epidermal growth factor receptor Idrissi Serhrouchni et al. Diagnostic Pathology 2013, 8:15 Page 3 of 4 http://www.diagnosticpathology.org/content/8/1/15 Figure 3 Tumor cells made clear cytoplasm with an hyperchromatic nucleus and a high mitotic activity (hematoxylin-eosin, original magnification: A, × 200, B, x 400). strongly decorated 81% of sweat gland tumors, but only diagnosis of adnexal cancer; in their study, none of the 17% of metastatic breast cancers (P=0.001). There was examples of metastatic adenocarcinoma to skin stained no significant difference between the skin tumors and for p63, whereas virtually all the adnexal carcinomas metastatic breast carcinoma when antibodies against es- were positive. trogen and progesterone receptors were used, but with These studies showed that immunohistochemistry progression of disease, androgen receptors (AR) are pre- does not distinguish cutaneous eccrine tumours from served with higher frequency than ER/PR in metastatic cutaneous metastases of breast carcinoma, in which case mammary carcinoma [13]. In another study comparing clinical and radiological correlation is critical [24]. benign, malignant primary eccrine and apocrine neo- Other differential diagnoses comprise neoplasms with plasms to metastatic breast carcinoma, only 3.5% of clear cell differentiation. These include trichilemmal car- these primary adnexal cancers demonstrated HER-2 cinoma, clear cell BCC and clear cell carcinomas meta- positivity, whereas 10–23% of the breast carcinomas static to the skin. With respect to the latter, the were positive [13,20].

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